Tongue, nail and conjunctiva were pink, temperature was 99.4 °F, pulse was 130/min., ... clubbing or edema feet. Investigations were as follows: ABG analysis ...
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Case Report
Tropical Pulmonary Eosinophilia : Masquerading as Eosinophilic Leukaemia Shrikant S. Somani, Resident, Asha N. Shah, HOD and Professor, Pooja V. Kothari, Resident, Harsha Jivarajani, Assistant Professor, Abhinav Jain, Resident — Department of Medicine, Civil Hospital, Ahmedabad. Abstract Benign conditions like Tropical Pulmonary Eosinophilia(TPE) can present with very high total count and Absolute Eosinophil Count (AEC) and can mimick malignancy. Diagnostic work up for TPE should be done in any patient presenting with pulmonary symptoms and eosinophilia. Though most case series on TPE report AEC in range of 3000 to upto 20,000, very rarely AEC can rise beyond 50,000. The following case is of TPE presenting with absolute eosinophil count of >70,000. Rapid response to Diethyl carbamazine is the rule in a confirmed case of TPE. Keywords tropical pulmonary eosinophilia, absolute eosinophil count Case Report An 18-year old male, Hindu, unmarried patient residing at Vadinath village, Jamnagar, Gujarat, India from a low socio- economic class working as a house painter presented in the emergency room with the chief complaints of: 1.
Low grade fever for 15 days
2.
Cough with expectoration for 12 days
3.
Dyspnoea on exertion for 7 days
The patient developed low grade fever without chills and rigors since 15 days which was persistent throughout the day and was relieved by taking antipyretic medications. Then after about 3 days he started coughing with scant whitish expectoration. Gradually, the intensity of cough increased. Since last 7 days, he developed gradually progressive dyspnoea on exertion, which was initially relieved at rest but for the last two days increased so that the patient had dyspnoea even on rest. There was no complaint of high spiking fever, rash, orthopnoea, PND, edema feet, hemoptysis, chest pain, palpitations, decreased urine output, anorexia, weight loss, vomiting, altered sensorium or seizures. Provisional Diagnosis: Acute infective/allergic bronchitis, viral pneumonia, Tuberculosis. Past History: There was no past history of similar complaints, Tuberculosis, asthma, allergy, drug reactions, unsafe sexual exposure or any other major illness. Personal History: Not significant. No addiction history. Family History: Was non significant. General Examination: he was conscious, cooperative, oriented to time, place and person, well built and well
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nourished. Tongue, nail and conjunctiva were pink, temperature was 99.4 °F, pulse was 130/min., blood pressure was 120/80 mm Hg, sclera was white, respiratory rate was 38/min. Respiratory system examination: Upper respiratory tract was normal. On inspection, breathing was shallow and rapid. There was in-drawing of lower rib spaces and use of accessory muscles. Trachea was central. Rest was normal. On palpation, we confirmed the inspectory findings. There was no disparity in the movement of chest wall on both sides and there was no change in tactile vocal fremitus. On percussion, lung and heart borders were normally located. The percussion note was resonant in all areas of lung. On auscultation, bilateral air entry was present with shortened inspiration and expiration due to tachypnoea. The pattern of breathing was bronchial type. Bilateral harsh breathing was evidenced by presence of bilateral inspiratory and expiratory rhonchi throughout the entire lung field which were more prominent anteriorly in the mammary region and posteriorly in the interscapular region. There were no crepitations. Vocal resonance was increased in the above mentioned areas. The other systemic (Cardiovascular, Central nervous system, Abdomen) examination was normal. There was no evidence of cyanosis, lymphadenopathy, clubbing or edema feet. Investigations were as follows: ABG analysis was suggestive of mild hypoxia with SPO2 - 86% and PaO264 mm Hg, PaCO2 -32 mmHg, pH- 7.37. Blood counts showed Hb – 12.9, TC – 94,200, DC- 9/16/74/1 (neutro, lympho, eosino, basophils), PC – 3.95 lac and P/S picture – normocytic hypochromic RBC, leukocytosis with eosinophilia, AEC -69,700. Renal and liver functions were normal. HIV and HbsAg were negative by ELISA. Fasting blood sugar was 109. Stool tests didn’t show any ova or parasites. ECG was normal and chest x-ray was suggestive of bilateral increased broncho vascular markings in the lower and middle zones suggestive of bronchitis. Sputum cytology was suggestive of plenty of eosinophils. Sputum for AFB and culture were negative.
Ultrasound abdomen showed no hepato spleenomegaly or lymphadenopathy. Ultrasound thorax didn’t show any effusion or underlying consolidation. 2-D echocardiography was normal with normal left ventricular size, systolic function and ejection fraction. Patient was planned for bone marrow biopsy to evaluate the eosinophilic leukocytosis and rule out malignancy. Meanwhile, patient was given high flow oxygen with ventimask and started on intravenous broad spectrum antibiotic- Inj. Coamoxiclav and oral anti-filarial drug Diethyl carbamazine(DEC). Patient also received nebulization with salbutamol, ipratropium and budesonide. Patient’s tachypnoea improved and >95% oxygen saturation was obtained within 24 hours of admission. By third day of admission patients symptoms drastically improved and and he was not requiring continous oxygen. Even the findings on auscultation improved significantly. Bone marrow biopsy was suggestive of marked proliferation of Eosinophilic precursors and Eosinophils. The differential count showed Eosinophils (72%), Late Normoblasts (12%), Myelocytes (7%), Lymphocytes and Polymorphs forming (2%) each. Erythroid series showed mild suppression. No blast or malignant cells were seen. Simultaneously done P/S picture was showing AEC of 76,320. IG E levels in blood was >2000 (normal 3000 eosinophils/ microL). Chest x-rays or CT scans may be normal but generally show increased bronchovascular markings. Tests of pulmonary function show restrictive abnormalities in most cases and obstructive defects in half. Characteristically, total serum IgE levels
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(10,000–100,000 ng/mL) and anti-filarial antibody titres are markedly elevated2.
eosinophilia, associated hypercellularity, and tissue damage, originally based on criteria of Chusid et al7.
In a study done on 17 patients of TPE presenting at the Toronto General Hospital, most common symptoms at presentation were shortness of breath (88%) and nocturnal cough (88%), whereas wheezing was the most common sign (88%). The eosinophil count was elevated in all patients and was >3 × 109eosinophils/L in 16 patients (94%). The median eosinophil count was 16.9 × 109 eosinophils/L. The median total leukocyte count was 24.3 × 109 leukocytes/L. In all 17 patients studied, the total leukocyte count was >60,000 in only one patient and AEC was >50,000 in only one patient3.
Another distinct entity presenting with high grade eosinophilia in young age group is M4E0 type of Acute Myelomonocytic leukaemia. This type is a associated with a specific genetic marker inv 16 and such cases have good prognosis. The bone marrow show promyelocytes, myelocytes with larger esoinophilic/purple violet granules, alongwith blasts8.
In our patient, the total leukocyte count on presentation was 94,200/ microL with 74% eosinophils i.e. AEC- 69,700, which increased to AEC -76,320 before DEC therapy was started. Eosinophilia is defined as the presence of >500 eosinophils/ microL of blood. Division of eosinophil count is arbitrary, but a mild eosinophilia can be regarded as less than 1500/ microL (1.5x109/liter), a moderate elevation as 1500 – 5000/ microL (1.5–5.0 x 109/liter), and a high count as greater than 5000/ microL (5.0 x 109/liter)4. The most common cause of eosinophilia worldwide is infection with helminthic parasites, which often can result in a very high eosinophil count. The other common causes of mild to moderate eosinophilia are allergic reaction to drugs and allergies such as hay fever, asthma, eczema, serum sickness, allergic vasculitis and pemphigus. Eosinophilia also occurs in collagen vascular diseases and malignancies (e.g., Hodgkin’s disease, mycosis fungoides, chronic myeloid leukemia, and cancer of the lung, stomach, pancreas, ovary or uterus). The most dramatic hypereosinophilic syndromes are Loeffler’s syndrome, tropical pulmonary eosinophilia, Loeffler’s endocarditis, eosinophilic leukemia, and idiopathic hypereosinophilic syndrome (50,000–100,000/ microL)5. The diagnostic algorithm for hypereosinophilia should include a bone marrow examination with cytogenetic studies and screening for the FIP1L1-PDGFRA fusion gene to rule out chronic eosinophilic leukemia/ hypereosinophilic syndrome for those patients whose workup is negative for secondary causes of eosinophilia6. Chronic Eosinophilic Leukaemia/Idiopathic Hypereosinophilic syndrome is defined as an unexplained peripheral blood proliferation of >1500 cells/microlitre of eosinophils for 6 months or more with bone marrow
Conclusion Tropical Pulmonary Eosinophilia is a rare and serious syndrome occurring in some individuals infected with lymphatic filarial species Wuchereria bancrofti and Brugia Malayi. It should always be considered in the differential diagnosis of a patient with pulmonary symptoms and blood eosinophilia particularly in tropical countries. Bone marrow examination must be done to rule out leukemia if the primary tests turn out to be negative. Early institution of DEC should be done in a confirmed case of TPE. References 1.
Ong R. K., Doyle R. L. —Tropical Pulmonary Eosinophilia. Chest. vol. 113(6):1673-1679, June 1998.
2.
Nutman T. B., Weller P. F. — Filarial and related infections, Harrison’s Principles of Internal Medicine, 17th edition, Mc Graw Hill. 211:1326.
3.
Boggild A. K., Keystone J. S., Kain K. C. — Tropical Pulmonary Eosinophilia: A Case Series in a Setting of Nonendemicity. Clinical Infectious Diseases. vol. 39 (8):1123-1128.
4.
Moqbel R., Odemuyiwa S. O., Lacy Paige, Adamko D. J. — The Human Eosinophil. Wintrobe’s Clinical Hematology, 12th edition, Lippincott Williams & Wilkins Philadelphia. vol. 1(10):215.
5.
Henry P. H., Longo D. L. — Enlargement of lymph nodes and spleen, Harrison’s Principles of Internal Medicine. 17th edition, Mc Graw Hill, 60:383.
6.
Gotlib J., Cools J., Malone J. M. 3rd, et al. — The FIP1L1PDGFR alpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnostics, classification, and management. Blood. 103: 2879-2891, 2004.
7.
George T. I. — Pathology of Myeloproliferative disorders. Wintrobe’s Clinical Hematology, 12th edition, Lippincott Williams & Wilkins, Philadelphia. vol. 4(84):1996.
8.
Singh T. — Atlas and Text of Hematology. Acute Leukaemia, 1st edition, Avichal Publishing Company, Sirmour. 6:151, 2010.
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